PRIONS Flashcards
Prions are derived from what words?
Proteins and Infections; infections and proteins or rogue proteins
Prions is coined by who ?
Stanley B. Prusiner (1982)
What are the term for the several neurodegenerative ds. caused by prions?
Transmissible spongiform encephalopathy
What are the characteristic lx. induced by prions?
- Spongiform degeneration; activation and proliferation of astrocytes and microglia in brain and spinal cord
What are the most infectious prion particles ?
14-28 PrP molecules
Size of the prions has been noted as small as ?
30 nm
Prions polymerase after ?
Proteinase-K digestion
This is the term for normal cellular proteins that have undergone pathological conformational change that occurs posttranslationally; protein in a misfolded form.
Prions
This is the abbreviation for prion protein.
PrP
This is the abbreviation for normal protein; normal cellular isoform of prion protein.
PrPc
This is the abbreviation form for abnormal isoform of the protein; derived from the scrapie isoform of the prion protein.
PrPSc
How do prions propagate?
- By transmitting a misfolded protein state.
- They induce properly folded proteins to convert into the disease-associated prion form
Give the disease of prions with the given data:
- C/S: : excitable, fine tremors of head and neck, intense pruritus, wool loss, emaciation,
weakness, weaving gait, staring eyes, ataxia, hindquarter paralysis, animals invariably die
Scrapie (Classical scrapie)
Give the disease of prions with the given data:
- Lesions and PrPSc accumulation occur in the dorsal motor nucleus of the vagus nerve in
the medulla
Scrapie (Classical scrapie)
> 1500 cases of iatrogenic scrapie followed the use of what vaccine?
Formalin-inactivated louping ill vaccine prepared from sheep brain
What is the incubation period of Scrapie?
2-5 yrs.
Give the disease of prions with the given data:
- Lesions and PrPSc accumulation occur in the cerebellum
Nor98 (Atypical Scrapie)
Give the disease of prions with the given data:
- PrPSc absent from lymphoid tissues
Nor98 (Atypical Scrapie)
Give the disease of prions with the given data:
- First recognized in UK in 1986
Bovine spongiform encephalopathy (mad cow ds.)
Give the disease of prions with the given data:
- Associated with feeding of meat-and-bone meal derived from ruminant meat or offal
(banned) - Onset is insidious with tremors, hyperesthesia, abnormal posture, hind limb ataxia, aggression, weight loss
Bovine spongiform encephalopathy (mad cow ds.)
Give the disease of prions with the given data:
- Characteristic lesions occur only in the brain and spinal cord of cattle (e.g. neuronal
vacuolation)
Bovine spongiform encephalopathy (mad cow ds.)
What age of cattle were most affected by mad cow ds.?
3-5 years of age
Give the disease of prions with the given data:
- C/S: hyper-irritability, ataxia, compulsive biting, somnolence (state of drowsiness or
sleepiness), coma and deat
Transmissible mink encephalopathy
The histological lesions of transmissible mink encephalopathy is the same with ____ in sheep.
scrapie
